HELLP Syndrome

HELLP SYNDROMEOleh :Lius Hariman (0510036)Yeremia Rerung (0510090)Maria Christina (0510099)Fenny Shuriana (0510126)Irvan Amadeo T. (0510177)

1

DATA PASIEN:

Nama: Erni NoveantyUmur: 30 thnBB : 63 KgTB : 160 cmBMI : 24.61 kg/m2Tgl msk : 20-12-10Diagnosa masuk: G2P1A0 6-7bln dan hipertensy urgency (keracunan?, eclampsia?)

2

Keluhan:G2P1AO hamil 6-7bln datang dengan kejang sejak kurang lebih 1 seperempat jam yg lalu, kejang seluruh tubuh, antar kejang terdapat keadaan sadar, setiap kejang 2-3 detik, hilang timbul, 4 jam yang lalu pasien mengalami penurunan kesadaran yang hilang timbul.Kemarin siang pasien mengeluh mual, muntah lalu berobat ke bidan dan diberi obat (tapi tidak tahu nama obat)

3

Riwayat kehamilan : kehamilan pertama normal

RPD : Hipertensi -, DM

4

KONDISI SAAT MASUK IGD:

Tgl 20/12/2010 Mulai periksa : 05.31 Tensi : 240/110mmhgNadi : 143x/mntSuhu : 37.4 derajat CKesadaran : deliriumTriage : merah

5

Pemeriksaan Fisik:Kepala : B/U simetrisMata : bulat isokor, diameter 3mm, RC -/-, konjunctiva anemis -/-, sklera ikterik -/-Bibir : bibir bawah oedem +Leher : KGB tidak tampak membesarThorax : B/P simetrisPulmo : VBS +/+, Rh +/+, Wh -/-Cor : BJM reg murmur Abdomen : cembung, gravidExtremitas : oedem -/-, akral hangatCRT 1.2 mg/dL Lactate dehydrogenase level > 600U/L Elevated liver function test result Serum aspartate amino transferase level > 70U/L Lactate dehydrogenase level >600 U/L Low platelet count Platelet count < 150 000/mm3

34

Platelet count appears to be the most reliable indicator of the presence of HELLP syndrome.

35

CLASSIFICATIONfull HELLP syndrome partial HELLP syndrome based on the number of abnormalities

Audibert F, Friedman SA, Frangieh AY, Sibai BM. Am J Obstet Gynecol 1996; 175:460-4. considered for delivery within 48 hourscandidates for more conservative management

36

CLASSIFICATIONon the basis of platelet count :class I, less than 50,000 per mm3 class II, 50,000 to less than 100,000 per mm3 class III, 100,000 to 150,000 per mm3

37

MANAGEMENT

DeliveryCorticosteroidsMagnesium sulphateHypotensive drugsBlood products

38

Restricted to addressing manifestations and side effects associated with this diseaseTreatment consists of bed rest, sedation, antihypertensive medications, and magnesium sulfate therapyInvasive arterial, central venous, and possibly pulmonary artery monitoring may be indicated in patients with severe hypertension, pulmonary edema, or refractory oliguriaDefinitive treatment is delivery of the fetus and placenta!

39

The treatment approach should be based on the estimated gestational age and the condition of the mother and fetus.

Prolongation of pregnancy, in theory, may be favourable for the foetus whereas it remains controversial whether maternal condition is further deteriorated by expectant management

Visser W, Wallenburg HC. Temporising management of severe pre-eclampsia with and without the HELLP syndrome. Br J Obstet Gynaecol 1995;102:111-7

40

ELIGIBILITY TO CONSERVATIVE MANAGEMENT

hypertension is controlled at less than 160/110 mm hg,Oliguria responds to fluid management .Elevated liver function values are not associated with right upper quadrant or epigastric pain.Class II III .(platelet count).>50000

41

The antenatal administration of dexamethasone (Decadron) in a high dosage of 10 mg intravenously every 12 hours has been shown to markedly improve the laboratory abnormalities associated with HELLP syndrome.

Steroids given antenatally do not prevent the typical worsening of laboratory abnormalities after delivery. However, laboratory abnormalities resolve more quickly in patients who continue to receive steroids postpartum.

EF, Bass D, Chauhan SP, Sullivan DL, Martin RW, Martin JN Jr. Am J Obstet Gynecol 1994;171:1148-53.

42

Corticosteroid therapy should be instituted in patients with HELLP syndrome who have a platelet count of less than 100,000 per mm3 .And should be continued until liver function abnormalities are resolving and the platelet count is greater than 100,000 per mm3

Magann EF, Perry KG Jr, Meydrech EF, Harris RL, Chauhan SP, Martin JN Jr. Am J Obstet Gynecol 1994;171:1154-8.

43

Intravenously administered dexamethasone appears to be more effective than intramuscularly adminstered betamethasone for the antepartum treatment of mothers with HELLP syndrome.

(Am J Obstet Gynecol 2001;184:1332-9.).

44

Administration of glucocorticoids increases the use of regional anesthesia in women with antepartum HELLP syndrome who have thrombocytopenia.

(Am J Obstet Gynecol 2002;186:475-9.).

45

Patients with HELLP syndrome should be treated prophylactically with magnesium sulfate to prevent seizures, whether hypertension is present or not.

46

Antihypertensive therapy should be initiated if blood pressure is consistently greater than 160/110 mm hg despite the use of magnesium sulfate. The goal is to maintain diastolic blood pressure between 90 and 100 mm hg.

47

The most commonly used antihypertensive agent has been hydralazine LabetololNifedipine

48

BLOOD PRODUCT

49

Between 38 -93 % of patients with HELLP syndrome receive some form of blood product. Patients with a platelet count greater than 40,000 per mm3 are unlikely to bleed. Patients who undergo cesarean section should be transfused if their platelet count is less than 50,000 per mm3 ,Prophylactic transfusion of platelets at delivery does not reduce the incidence of postpartum hemorrhage or hasten normalization of the platelet count. . Patients with DIC should be given fresh frozen plasma and packed red blood cells.

50

ANASTHESIA CONSIDERATION

51

Pain relief with intravenous narcotics and local anesthesia is acceptable but certainly not optimal for pain control.Epidural anesthesia has been controversial but it is the technique of choice when it can be accomplished safely. Insertion of an epidural catheter is generally safe in patients with a platelet count greater than 100,000 per mm3. General anesthesia can be used when regional anesthesia is considered unsafe.Portis R, Jacobs MA, Skerman JH, Skerman EB. HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) pathophysiology and anesthetic considerations. AANA J 1997;65:37-47.

52

Patients with severe disease require stabilization prior to the administration of any anesthetic. Hypertension should be controlled and hypovolemia corrected. In the absence of coagulopathy, continuous epidural anesthesia is the anesthetic of choice for most patients with PIHPlatelet count and coagulation profile should be checked prior to initiation of regional anesthesia in patients with severe PIHIntra-arterial blood pressure monitoring indicated for severe hypertension. Central venous line may guide volume replacement. Pulmonary artery may also be indicatedMagnesium sulfate potentiates muscle relaxants

53

COMPLICATIONS

54

The mortality rate for women with HELLP syndrome is approximately 1.1 % From 1 to 25 % of affected women develop serious complications such as DIC, placental abruption, adult respiratory distress syndrome, hepatorenal failure, pulmonary edema, subcapsular hematoma and hepatic rupture. A significant percentage of patients receive blood products. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol 1993;169:1000-6.

55

Infant morbidity and mortality rates range from 10 to 60 %, depending on the severity of maternal disease.

Infants affected by HELLP syndrome are more likely to experience intrauterine growth retardation and respiratory distress syndrome.

Dotsch J, Hohmann M, Kuhl PG. Neonatal morbidity and mortality associated with maternal haemolysis, elevated liver enzymes and low platelets syndrome. Eur J Pediatr 1997;156:389-91.

56

Once the diagnosis of HELLP syndrome has been established, the best markers to follow are the maternal lactate dehydrogenase level and the maternal platelet count

57

The laboratory abnormalities in HELLP syndrome typically worsen after delivery and then begin to resolve by three to four days postpartum.Martin JN Jr, Blake PG, Perry KG Jr, McCaul JF, Hess LW, Martin RW. The natural history of HELLP syndrome: patterns of disease progression and regression. Am J Obstet Gynecol 1991;164(6 pt 1):1500-9.

58

The incidence of hemorrhagic complications is higher when platelet counts are < 40,000 per mm3

59

Patients with HELLP syndrome who complain of severe right upper quadrant pain, neck pain or shoulder pain should be considered for hepatic imaging regardless of the severity of the laboratory abnormalities, to assess for subcapsular haematoma or rupture

60

SUMMARYPreeclampsia is a multi-system disorder producing maternal morbidity and mortalityMagnesium sulfate remains the agent most often used to prevent seizures in the preeclamptic patient. If convulsions occur, they should be immediately treated with intravenous benzodiazepines or thiopentalIndications for invasive monitoring include: severe oliguria, pulmonary edema, and severe hypertension unresponsive to aggressive pharmacologic managementEpidural anesthesia provides numerous maternal and fetal benefits to the preeclamptic patient

61